摘要
目的:肾黏液小管和梭形细胞癌(mucinous tubular and spindle cell carcinoma,MTSCC)是一种罕见的肾肿瘤,容易误诊为其他类型肿瘤。本研究探讨MTSCC的临床病理特征、免疫表型、分子病理特点、鉴别诊断及预后,旨在提高病理医师对该肿瘤的认识。方法:回顾性分析2012年1月至2023年12月西安市第三医院和陕西省人民医院病理科外检存档的6例MTSCC的临床病理特征并复习相关文献。结果:6例患者中,男1例,女5例,年龄为52~78(中位数63)岁。临床表现为无症状3例,腰部不适2例,血尿1例。4例获得随访,随访14~70个月,其中1例死于其他疾病,余3例未见肿瘤复发和转移。镜下均由紧密排列小管、梭形细胞及黏液样间质构成,小管状区域与梭形细胞区域移行,梭形细胞呈条束状排列;肿瘤细胞形态温和、大小较一致,核仁不清,核分裂象少见,间质可见少量淋巴细胞浸润。肿瘤细胞表达广谱细胞角蛋白(pan-cytokeratin,pan-CK)(4/4)、细胞角蛋白(cytokeratin,CK)7(4/4)、配对盒基因8(paired box gene 8,PAX8)(4/4)、α-甲基酰基辅酶A消旋酶(alpha-methylacyl-CoA recemase,AMACR)(4/4)、上皮膜抗原(epithelial membrance antigen,EMA)(5/5)、波形蛋白(vimentin)(6/6),不表达共同急性淋巴母细胞白血病抗原(common acute lymphocyte leukemia antigen,CALLA or CD10)(0/5)、肾母细胞肿瘤蛋白1(Wilm’s tumor 1,WT1)(0/3)、自然杀伤细胞(nature killer cell,NK or CD57)(0/2)、转录因子增强子3(transcription factor E3,TFE3)(0/6),肿瘤蛋白P53(tumor protein 53,TP53)均呈野生型表达模式,细胞增殖抗原Ki-67增殖指数为5%~30%。结论:肾黏液小管和梭形细胞癌是一种较罕见的肿瘤,其诊断需要结合组织学形态及免疫组织化学检测,并需与乳头状肾细胞癌、集合管癌、后肾腺瘤等进行鉴别。
Objective:Mucinous tubular and spindle cell carcinoma(MTSCC)is a rare renal tumor that can be easily misdiagnosed as other tumor types.This study aims to investigate the clinicopathological characteristics,immunophenotype,molecular pathology,differential diagnosis,and prognosis of MTSCC,with the goal of improving diagnostic awareness among pathologists.Methods:A retrospective analysis was conducted on the clinicopathological features of 6 MTSCC cases archived from January 2012 to December 2023 at the pathology departments of Xi’an Third Hospital and Shaanxi Provincial People’s Hospital.Relevant literature was also reviewed.Results:Of the 6 patients,1 was male and 5 were female,aged 52-78 years(median,63 years).Clinical presentations included asymptomatic(n=3),flank discomfort(n=2),and hematuria(n=1).Four patients were followed for 14-70 months;one died of unrelated disease,and the remaining three showed no tumor recurrence or metastasis.Microscopically,tumors consisted of closely packed tubules,spindle cells,and mucinous stroma,with transitional areas between tubular and spindle cell regions.Spindle cells were arranged in fascicles.Tumor cells exhibited mild,uniform morphology,inconspicuous nucleoli,and rare mitotic figures;sparse lymphocytic infiltration was noted in the stroma.Immunohistochemically,tumor cells were positive for pan-cytokeratin(CK)(4/4),cytokeratin 7(CK)7(4/4),paired box gene 8(PAX8)(4/4),alpha-methylacyl-CoA racemase(AMACR)(4/4),epithelial membrane antigen(EMA)(5/5),and vimentin(6/6),but negative for common acute lymphocyte leukemia antigen(CALLA/CD10)(0/5),Wilms tumor 1(WT1)(0/3),nature killer cell antigen(NK/CD57)(0/2),and transcription factor E3(TFE3)(0/6).Tumor protein 53(TP53)showed a wild-type expression pattern.The Ki-67 proliferation index ranged from 5%to 30%.Conclusion:MTSCC is a rare renal neoplasm whose diagnosis requires integration of histological morphology and immunohistochemistry,and should be differentiated from papillary renal cell carcinoma,collecting duct carcinoma,and metanephric adenoma.
作者
苟思琪
方航荣
王娟红
魏威
段瑛
左伟晨
姚丽
陈琳
李艺洁
GOU Siqi;FANG Hangrong;WANG Juanhong;WEI Wei;DUAN Ying;ZUO Weichen;YAO Li;CHEN Lin;LI Yijie(Department of Pathology,Xi’an No.3 Hospital,Affiliated Hospital of Northwest University,Xi’an 710021;Department of Pathology,Shaanxi Provincial People’s Hospital,Xi’an 710068,China)
出处
《临床与病理杂志》
2025年第5期628-634,共7页
Journal of Clinical and Pathological Research
基金
西安市科技计划项目(23YXYJ0001)。
关键词
肾黏液小管和梭形细胞癌
肾肿瘤
细胞角蛋白
波形蛋白
病理诊断
mucinous tubular and spindle cell carcinoma
renal tumor
cytokeratin
vimentin
pathological diagnosiss
